Nazha Aziz, Prebet Thomas, Gore Steven, Zeidan Amer M
Leukemia Program, Department of Hematology and Oncology, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, USA.
Section of Hematology, Department of Internal Medicine, Yale University, and Yale Comprehensive Cancer Center, New Haven, CT, USA.
Blood Rev. 2016 Sep;30(5):381-8. doi: 10.1016/j.blre.2016.04.006. Epub 2016 Apr 25.
Chronic myelomonocytic leukemia (CMML) is a unique disease entity with overlap components of both myelodysplastic syndrome and myeloproliferative neoplasms. CMML is a clonal hematopoietic stem cell neoplasm characterized by monocytosis, cytopenias, and extramedullary manifestations such as splenomegaly. The disease is rare and has undergone revisions in its classification. We review the recent classification strategies as well as diagnostic criteria, focusing on the new insights into the genetic alterations and unique pathophysiology of the disease. We also discuss the latest molecular characterization of the disease, including how molecular factors affect current prognostic models. Finally, we focus on available treatment strategies, with a special emphasis on experimental and forthcoming therapies.
慢性粒单核细胞白血病（CMML）是一种独特的疾病实体，兼具骨髓增生异常综合征和骨髓增殖性肿瘤的重叠成分。CMML是一种克隆性造血干细胞肿瘤，其特征为单核细胞增多、血细胞减少以及脾肿大等髓外表现。该疾病较为罕见，其分类也历经了修订。我们回顾了近期的分类策略以及诊断标准，重点关注该疾病基因改变和独特病理生理学的新见解。我们还讨论了该疾病的最新分子特征，包括分子因素如何影响当前的预后模型。最后，我们聚焦于可用的治疗策略，特别强调实验性和即将出现的疗法。
